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Causes and clinical manifestations of primary adrenal insufficiency in children

Patricia A Donohoue, MD
Section Editor
Mitchell E Geffner, MD
Deputy Editor
Alison G Hoppin, MD


Adrenal insufficiency is defined by the impaired synthesis and release of adrenocortical hormones. It is classified based upon the mechanism:

Primary adrenal insufficiency results from disease intrinsic to the adrenal cortex (table 1). These patients may present with glucocorticoid deficiency, with or without deficiencies of mineralocorticoids and adrenal androgens.

Central adrenal insufficiency is caused by impaired production of adrenocorticotropic hormone (ACTH). It can be caused by either pituitary disease that impairs release of ACTH, or by interference with corticotropin releasing hormone (CRH) production from the hypothalamus. These patients have only glucocorticoid deficiency and not mineralocorticoid deficiency. In addition, they may have other pituitary hormone deficiencies, depending on the underlying central nervous system disease.

The causes and clinical manifestations of primary adrenal insufficiency will be discussed in this topic review. The central (secondary and tertiary) causes of adrenal insufficiency are discussed elsewhere in the program (see "Causes and clinical manifestations of central adrenal insufficiency in children"). The diagnosis and treatment of adrenal insufficiency is discussed separately. (See "Diagnosis of adrenal insufficiency in children" and "Treatment of adrenal insufficiency in children".)


The signs and symptoms of primary adrenal insufficiency vary depending on what class(es) of hormone are deficient and the severity of the defect(s). The adrenal hormones are divided into glucocorticoids (eg, cortisol), mineralocorticoids (eg, aldosterone), and adrenal androgens (eg, dehydroepiandrosterone [DHEA]).

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Literature review current through: Nov 2017. | This topic last updated: Dec 06, 2017.
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